Purpose <p>Spinal cord compression (SCC) is a rare but critical oncological emergency in children and may represent the initial manifestation of an underlying malignancy. Data focusing specifically on this presentation remain limited. This study evaluated clinical characteristics, neurological outcomes, and survival in pediatric patients presenting with SCC as the initial symptom.</p> Methods <p>In this retrospective study, we included pediatric patients who presented with their first symptom of SCC between 1998 and 2020. Clinical characteristics, symptom presentation, radiologic localization, pathology, treatment, neurological outcomes, and survival were assessed. Neurological outcomes were classified as complete, partial, or none.</p> Results <p>Forty patients were included, representing 2.3% of pediatric oncology cases. Median age was 8.9 years and median symptom duration was 20 days. Extramedullary–extradural lesions were most frequent (67.5%), while intramedullary localization was significantly associated with neurological sequelae. Decompressive surgery was performed in 75% of patients and was linked to a markedly lower rate of persistent sequelae (33.3% vs. 80%). Presentation beyond 28 days increased the risk of permanent neurological sequelae nearly 16-fold. Symptom duration showed strong predictive accuracy (AUC = 0.865). Overall survival varied significantly by histopathological diagnosis.</p> Conclusions <p>Pediatric patients who present with SCC as the initial sign of cancer are at considerable risk of long-term neurological morbidity. Disease duration and intramedullary location correlated with poor neurological outcome; the effect of decompressive surgery should be taken into account in individualized decision-making. Such results highlight the need for early detection, prompt MRI, and multidisciplinary intervention.</p> Clinical trial registration <p>This study was conducted retrospectively. Therefore, clinical trial registration was not required. Clinical trial number: not applicable.</p>